Granulomatosis with polyangiitis presenting headache: A case report and review of literature

• Published in: Medicine (Baltimore) 2024-01, Vol.103 (2), p.e36972-e36972
• Main Authors: Li, Zhao, Zhang, Qiushuang, Wang, Xuebin, Shi, Fang
• Format: Article
• Language: English
• Subjects: Clinical Case Report; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - diagnosis; Granulomatosis with Polyangiitis - drug therapy; Headache - etiology; Hearing Loss - complications; Humans; Meningitis - etiology; Rituximab - therapeutic use
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000036972

Central nervous system involvement is a rare manifestation of active-phaselocalized Granulomatosis with polyangiitis (GPA). In hypertrophic dura meningitis, GPA with headache is typical. In this case, cerebral magnetic resonance (MR) enhancement revealed no meningeal thickening, to our knowledge, this manifestation had not been found previously. The patient presented to the Rheumatology and Immunology Clinic with severe headache and hearing loss, and central nervous system granulomatosis with polyangiitis was confirmed after a series of examinations. The patient had no significant effect after treatment with cyclophosphamide (CTX), but after the use of rituximab, the headache and hearing loss were significantly improved, and laboratory indicators returned to normal levels. We comprehensively screened for craniocerebral infection and malignant tumors, diagnosed central nervous system granulomatosis with polyangiitis. We gave sequential treatment of rituximab. All indicators are mostly back to normal when the patient was monitored at the outpatient clinic. GPA and severe headache are more prevalent in hypertrophic dura meningitis, but the patient early headache could not be explained by hypertrophic dura meningitis or localized granulomatous lesions that invaded the central nervous system. Patients with severe headaches likely have vascular inflammation and local bone destruction at the base of the skull.